Phenotypes associated with this allele

• Allele Symbol: Ush1g^tm1.2Ugds
• Allele Name: targeted mutation 1.2, Unite de Genetique des Deficits Sensoriels
• Allele ID: MGI:4950014
• Summary: 1 genotype

Jump to	Allelic Composition	Genetic Background	Genotype ID
hm1	Ush1g^tm1.2Ugds/Ush1g^tm1.2Ugds	involves: 129S1/SvImJ * BALB/c * C57BL/6 * C57BL/6J	MGI:4950048

Genotype
MGI:4950048

hm1

• Allelic Composition: Ush1g^tm1.2Ugds/Ush1g^tm1.2Ugds
• Genetic Background: involves: 129S1/SvImJ * BALB/c * C57BL/6 * C57BL/6J

• ♀: phenotype observed in females
• ♂: phenotype observed in males
• N: normal phenotype

hearing/vestibular/ear

abnormal inner hair cell stereociliary bundle morphology ( J:171218 )

• disorganization of stereocilia reminiscent of that in mice homozygous for Ushg1^js

abnormal outer hair cell stereociliary bundle morphology ( J:171218 )

• disorganization of stereocilia reminiscent of that in mice homozygous for Ushg1^js

• bundles are often fragmented in two or more clumps of stereocilia

abnormal outer hair cell kinocilium location or orientation ( J:171218 )

• often mispositioned

abnormal vestibular hair cell stereociliary bundle morphology ( J:171218 )

• some stereocilia bundles are fragmented

absent cochlear microphonics ( J:171218 )

abnormal hair cell mechanoelectric transduction ( J:171218 )

• maximal amplitudes of mechanoelectric transduction (MET) currents are markedly reduced in inner and outer hair cells at P5

• average sensitivity of the MET currents to hair bundle displacement is decreased by about 50% in both inner and outer hair cells

• at P1 in utricular hair cells of the vestibule with intact stereocilia bundles the maximal amplitudes of MET currents are about 30% that of controls

increased or absent threshold for auditory brainstem response ( J:171218 )

• absent at all sound frequencies even at the highest intensity (105 dB SPL) tested

absent distortion product otoacoustic emissions ( J:171218 )

nervous system

abnormal inner hair cell stereociliary bundle morphology ( J:171218 )

• disorganization of stereocilia reminiscent of that in mice homozygous for Ushg1^js

abnormal outer hair cell stereociliary bundle morphology ( J:171218 )

• disorganization of stereocilia reminiscent of that in mice homozygous for Ushg1^js

• bundles are often fragmented in two or more clumps of stereocilia

abnormal outer hair cell kinocilium location or orientation ( J:171218 )

• often mispositioned

abnormal vestibular hair cell stereociliary bundle morphology ( J:171218 )

• some stereocilia bundles are fragmented

absent cochlear microphonics ( J:171218 )

abnormal hair cell mechanoelectric transduction ( J:171218 )

• maximal amplitudes of mechanoelectric transduction (MET) currents are markedly reduced in inner and outer hair cells at P5

• average sensitivity of the MET currents to hair bundle displacement is decreased by about 50% in both inner and outer hair cells

• at P1 in utricular hair cells of the vestibule with intact stereocilia bundles the maximal amplitudes of MET currents are about 30% that of controls

cellular

abnormal outer hair cell kinocilium location or orientation ( J:171218 )

• often mispositioned